Healthcare Provider Quick Facts
The following medical information is provided as a general resource only. It is not intended to be used for patient education. It does not create any patient-physician relationship and should not be used as a substitute for professional diagnosis and treatment.
ATS & JRS LAM Clinical Practice Guidelinesplus
Download the ATS/JRS 2016 LAM Clinical Practice Guidelines here.
Download the ATS/JRS 2017 LAM Clinical Practice Guidelines here.
Download both Guidelines with a summary page and clinical diagnosis algorithm here.
These guidelines synthesize the evidence for emerging advancements in lymphangioleiomyomatosis (LAM) and then use that evidence to formulate recommendations pertaining to the diagnosis and treatment of patients with LAM. The intent of these guidelines is to empower clinicians to apply the recommendations in the context of the values and preferences of individual patients and to tailor their decisions to the clinical situation at hand.
FDA Approved Treatmentplus
Rapamycin (also known as sirolimus) has been shown to slow or even stop the progression of LAM in some patients. On May 27, 2015, the FDA approved the use of Rapamycin for use in patients with LAM as a result of the MILES Clinical Trial.
Download the Efficacy and Safety of Sirolimus in Lymphangioleiomyomatosis (MILES Trial) article from the New England Journal of Medicine here.
Global Clinic Networkplus
LAM Clinics are strategically located in hospitals or medical centers across the world where there are clinicians and researchers who have an interest in LAM and who value research in rare lung diseases. In order to become a LAM Foundation approved LAM Clinic, an application must be submitted and reviewed by the Foundation’s LAM Clinic Committee before being accepted into the network.
LAM clinic care teams are also interested in improving the care and treatment of patients with other rare lung diseases, and are often referred to as LAM & Rare Lung Disease Clinics. Rare lung conditions of interest include: pulmonary Langerhans cell histiocytosis, pulmonary aveolar proteinosis, sarcoidosis, Sjogren’s syndrome, Alpha-1 antitrypsin deficiency, and pulmonary fibrosis, among others.
Download a list of all US LAM Clinics here.
Download a list of all International LAM Clinics here.
Free Online Certified CME/MOCplus
Lymphangioleiomyomatosis Evidence Based Practice, a new Continuing Medical Education (CME) Enduring Material activity, is now available through the joint providership of the University of Cincinnati and the RLDC for AMA PRA Category 1 Credits™ (1.00 hours), Non-Physician Attendance (1.00 hours), ABIM MOC Part 2 (1.00 hours). The course reviews the 2016 and 2017 ATS/JRS Clinical Practice Guidelines in addition to sharing clinical pearls from experienced LAM Clinic Directors.
This activity has been designed to meet the educational needs of pulmonologists, obstetricians and gynecologists, emergency room physicians, family medicine physicians, internal medicine physicians, nurse practitioners and physician assistants, and respiratory therapists involved in the management of patients with LAM. Clinicians can take the course and receive continuing education credits at no cost, for both CME and maintenance of certification (MOC).
Jointly provided by the University of Cincinnati and the Rare Lung Disease Consortium, this activity is supported by an independent educational grant from the UNC Eshelman Institute for Innovation as well as contributions from The LAM Foundation and the Rare Lung Disease Consortium.
To participate in this activity, please visit https://uc.cloud-cme.com/aph.aspx?EID=20754&P=3000&CaseID=2410.
About Lymphangioleiomyomatosis (LAM)
Lymphangioleiomyomatosis (LAM) is a rare lung disease in women that results in diffuse cystic changes in the lung parenchyma. It is associated with recurrent pneumothoraces, chylous pleural effusions, progressive respiratory failure and abdominal tumors, including lymphangiomyomas or angiomyolipomas, which can spontaneously hemorrhage and result in pain, anemia and hypotension.
Here are some insights for treating patients with LAM in the ER:
- Pneumothorax should generally be managed with chest tube drainage followed by pleurodesis on the first event.
- Cystic changes due to LAM are often not apparent on chest X ray. High-resolution CT scanning of the chest is the most definitive imaging test.
- Pleural effusions usually represent chylothorax. This may be verified by thoracentesis and assessment of pleural fluid triglycerides and cholesterol.
- Renal angiomyolipomas may bleed spontaneously producing flank pain, hematuria, hypotension and anemia.
- Lymphangiomyomas may present as low-density abdominal lesions that mimic lymphoma or other malignancies.
- If the patient is to undergo anesthesia, the anesthesiologist should be advised that the patient is at risk for a pneumothorax with positive pressure ventilation.
- RAPAMUNE® (sirolimus) is an FDA approved drug to treat LAM. Special precautions should be taken for patients using sirolimus as it can impair wound healing in the event of urgent surgery. Sirolimus can also produce lung injury, immunosuppression, mouth ulcers, peripheral edema, acne like lesions and elevations of cholesterol.
The ICD 10 Code for LAM is J84.81.
For additional information or for a referral to a LAM specialist, contact The LAM Foundation at 513.777.6889.
This content was created for general informational purposes only. The content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.